摘要目的 探讨抗接触蛋白相关蛋白-2(contactin associated protein-like 2,CASPR2)抗体相关脑炎患者的临床特点、辅助检查、治疗及预后情况.方法 对2015年3月至2018年4月郑州大学人民医院神经内科收治的11例抗CASPR2抗体相关脑炎患者的临床资料进行回顾性分析.结果 11例患者的发病年龄为20～74(35.6±19.4)岁,其中8例为女性.主要临床表现有:边缘性脑炎症状者7例,其中癫痫发作者6例,记忆力减退者4例,精神行为异常者2例;外周神经高兴奋性者4例;神经性疼痛者4例;自主神经功能障碍者6例,其中便秘5例,窦性心动过速3例,多汗2例,小便困难2例;合并睡眠障碍者7例;合并体重减轻者4例;合并小脑症状者2例;合并低钠血症者2例.11例患者中有2例头颅MRI异常,表现为海马或内侧颞叶的异常信号.有6例患者行18F-氟代脱氧葡萄糖PET-CT检查,其中3例存在异常,表现为内侧颞叶或大脑皮质的代谢增高.1例患者胸部增强CT和PET-CT提示有胸腺瘤.11例患者均接受免疫治疗,治疗后症状均有不同程度的改善.对其中9例患者随访6～24个月,有3例患者复发,再次免疫治疗有效.结论 抗CASPR2抗体相关脑炎主要表现为边缘性脑炎、外周神经高兴奋性、神经性疼痛、自主神经功能障碍、失眠等,免疫治疗有效,可有复发.

abstractsObjective To explore the clinical features,auxiliary examinations,therapies and prognoses of patients with antibodies against contactin-associated protein-like 2 (CASPR2).Methods The clinical data of 11 anti-CASPR2 encephalitis patients who were admited to the People's Hospital of Zhengzhou University from March 2015 to April 2018 were retrospectively analyzed.Results The age of these 11 cases was (35.6± 19.4) years (ranged 20-74 years),and eight cases were females.There were seven cases with limbic encephalitis which included six cases of epilepsy,four cases of memory impairment,two cases of mental and behavioral abnormalities.Four cases had peripheral nerve hyperexcitability.Four cases had neuropathic pain.There were six cases with autonomic dysfunction including five cases of constipation,three cases of tachycardia,two cases of hyperhidrosis,two cases of urinary disorder.Seven cases had sleep disorder.Four cases had weight loss.Two cases showed cerebellar symptoms and two cases had hyponatremia.Magnetic resonance imaging scan of the brain showed abnormal signal in two cases,mainly involved medial temporal lobe and the hippocampus.Six cases underwent 18F-fluorodeoxyglucose positron emission tomography-computed tomography (PET-CT) examination,and three cases showed abnormalities,including two with temporal hypermetabolism and one with cortical hypermetabolism.Chest enhanced CT and PET-CT showed thymoma in one case.All cases received immunotherapy,and after treatment their symptoms were improved.Long-term follow-up was performed in nine cases,and three cases relapsed.Conclusions The major clinical manifestations of anti-CASPR2 encephalitis were limbic encephalitis,peripheral nerve hyperexcitability,neuropathic pain,autonomic dysfunction,insomnia and so on.Immunotherapy was effective and some patients may have recurrence.